ArticleRMD open2023
Interstitial lung disease with and without progressive fibrosing phenotype in patients with idiopathic inflammatory myopathies: data from a large multicentric cohort.
Article in RMD open, 2023. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 26 papers.
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Who cites it
26 citing papers in PubMed, 37 citations in OpenAlex.
- Flares and definition of worsening in inflammatory myopathies: a narrative review of the literature.Rheumatology (Oxford, England) · 2026Review
- Epidemiology of idiopathic inflammatory myopathies: a population-based cohort in England, 2002-2021.RMD open · 2026Article
- High frequency of interstitial lung disease in Indian patients with adult- onset idiopathic inflammatory myopathy: Insights from a prospective observational cohort.Clinical rheumatology · 2026Observational
- Myocarditis in idiopathic inflammatory myopathies: serologic and clinical correlates in a single-center registry.Rheumatology international · 2026Article
- Preliminary nomogram model for predicting respiratory failure of patients with idiopathic inflammatory myopathies.Scientific reports · 2026Article
- Early detection and risk stratification in autoimmune-related interstitial lung disease: a state-of-the-art review.Respiratory research · 2026Review
- Distinct phenotypes and prognosis between Jo-1 and non-Jo-1 subtypes in anti-synthetase syndrome: a retrospective cohort study from China.Clinical rheumatology · 2026Article
- Distinct phenotypic clusters associated with progressive pulmonary fibrosis in anti-synthetase syndrome-associated interstitial lung disease.Frontiers in immunology · 2026Article
- Type I interferon pathway activation in connective tissue disease associated interstitial lung disease.Frontiers in immunology · 2026Review
- Nerandomilast attenuates idiopathic inflammatory myopathy-associated interstitial lung disease via inhibiting proliferation and differentiation of B cells.Frontiers in immunology · 2026Article
- A serum biomarker-based index for predicting progressive pulmonary fibrosis in patients with anti-synthetase syndrome: the LOCCM index.Respiratory research · 2025Article
- High-Resolution CT Findings in Interstitial Lung Disease Associated with Connective Tissue Diseases: Differentiating Patterns for Clinical Practice-A Systematic Review with Meta-Analysis.Journal of clinical medicine · 2025Review
- Advancements in mesenchymal stromal cells for treating connective tissue disease-associated interstitial lung disease: from basic research to clinical application.Stem cell research & therapy · 2025Review
- Serum ferritin as a specific biomarker of anti-MDA5-interstitial lung disease: a multicenter, case-control study in observational and validation datasets.Rheumatology international · 2025Observational
- Integration of clinical and serological biomarkers in a nomogram for predicting interstitial lung disease in idiopathic inflammatory myopathies.BMC rheumatology · 2025Article
- Comparison of Chest High-Resolution Computed Tomography Findings in Patients with Anti-Melanoma Differentiation-Associated Gene 5 Antibody-Positive and Antibody-Negative Progressive Pulmonary Fibrosis with Polymyositis/Dermatomyositis.Journal of clinical medicine · 2025Article
- Patients with anti-SAE+ dermatomyositis display refractory and difficult-to-treat skin manifestations: case series from two Italian cohorts and review of literature.Frontiers in immunology · 2025Review
- Efficacy of anifrolumab on skin, joint and lung involvement in anti-MDA5 positive dermatomyositis: a case report.Frontiers in immunology · 2025Article
- Rationale for phosphodiesterase-4 inhibition as a treatment strategy for interstitial lung diseases associated with rheumatic diseases.RMD open · 2024Review
- A Practical Multidisciplinary Approach to Identifying Interstitial Lung Disease in Systemic Autoimmune Rheumatic Diseases: A Clinician's Narrative Review.Diagnostics (Basel, Switzerland) · 2024Review
Corrections and comments
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Authors and funding
24 authors at 6 institutions in 3 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
objectivesPatients with connective tissue diseases can develop interstitial lung disease (ILD), leading to a progressive fibrosing ILD (PF-ILD) phenotype in some cases. We aimed to investigate the occurrence of PF-ILD in idiopathic inflammatory myopathies (IIMs), and factors potentially predicting this phenotype. Secondary aims were to assess the radiological pattern and factors associated with IIMs-ILD.
methodsPatients with IIMs from our multicentric prospective cohort were retrospectively evaluated. Data were recorded at IIMs and ILD diagnosis, and during follow-up. Patients with ILD were classified according to the predominant high-resolution CT (HRCT) pattern: non-specific interstitial pneumonia (NSIP), usual interstitial pneumonia (UIP) and organising pneumonia (OP). PF-ILD was defined according to the 2022 American Thoracic Society (ATS), European Respiratory Society (ERS), Japanese Respiratory Society (JRS) and Latin American Thoracic Society (ALAT) guidelines. Univariate and multivariate analyses were performed to identify factors associated to ILD and to PF-ILD.
resultsOf 253 patients with IIMs, 125 (49%) had ILD: 99 (78%) at IIMs diagnosis and 26 (22%) during follow-up (21/26 within 5 years). Multivariate analysis identified anti-Jo-1, anti-MDA5, anti-Ro52, high score on manual muscle test, mechanic's hands and Raynaud's phenomenon as independently associated with ILD. The predominant HRCT pattern was NSIP (50% of patients), followed by UIP (28%) and OP (22%). At 1-year follow-up, PF-ILD occurred in 18% of IIMs-ILD. PF-ILD was predicted by anti-MDA5, heliotropic rash, xerostomia and xerophthalmia at univariate but not at multivariate analysis.
conclusionPatients with IIM should be carefully screened for ILD at IIMs diagnosis and yearly during follow-up. All patients with IIMs-ILD should be carefully monitored to capture ILD progression since a consistent proportion of them are expected to develop PF-ILD.
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